Giovambattista De Aloe

Rothmund-Thomson Syndrome (Thomson-Type) and Myelodysplasia

Rothmund–Thomson syndrome (RTS) is a genetic disease characterized by developmental abnormalities and poikilodermatous skin changes that appear in infancy. An association with myelodysplastic syndromes is rarely reported in RTS, even though impairment of immune function and recurrent infections are described in the literature. A case of Thomson‐type RTS in a 14‐year‐old girl with trilinear myelodysplasia is presented. The patient was kept under hematologic surveillance for myelodysplastic syndrome. Bone marrow transplantation was considered unnecessary at present.

Thalidomide in the Treatment of Kaposi’s Sarcoma

Background: Kaposi’s sarcoma (KS) is a lymphangioproliferative tumour. Therapy of KS depends on the characteristics of the disease, especially area and growth rate of lesions, and patient condition. Currently symptomatic resectable lesions are excised, whereas more advanced disease and unresectable lesions are treated with radiotherapy. If a large area or internal organs are affected or other treatments fail, chemotherapy is used. Recently some authors have reported their encouraging experience in the use of thalidomide in patients with AIDS-related KS. Objective: To evaluate the efficacy of thalidomide in 3 patients with non-AIDS-related KS. Methods: Two patients with classic widespread cutaneous and 1 with iatrogenic cutaneous and visceral KS were treated with thalidomide (100 mg/day) for 12 months. Results: In all 3 patients partial remission was evident after 4 months of thalidomide therapy; in 2 out of 3 complete remission was achieved after 12 months of treatment. Conclusions: Our results seem to confirm the utility of thalidomide in the treatment of non-AIDS-related KS.

Congenital Subcutaneous Granuloma Annulare

Abstract:  Granuloma annulare is a palisading granulomatous skin disease which may be generalized, localized, perforating, or subcutaneous. Subcutaneous granuloma annulare is self‐limiting, affecting infants and children, with typical postnatal onset. Here we report a patient with congenital clinical manifestations.

Extracorporeal photopheresis in graft-versus-host disease

Graft‐versus‐host disease (GvHD) is a common and often serious complication of hematopoietic stem cell transplantation. There are two major forms of GvHD: an acute form which develops in the first 100days after HSCT, and a chronic form which develops later. Chronic GvHD is a multiorgan syndrome with many features of autoimmune diseases, such as sclerodermatous skin changes, cholestasis, pulmonary fibrosis, xerostomia, oral ulcerations, myositis and fasci‐itis. Unlike acute GvHD which is characterized by acute alloreactivity,the etiology of chronic GvHD is controversial and is believed to be either an extension of acute GvHD and/or a result of dysfunctional immune reconstitution with generation of autoantibodies and autoreactive T‐cell clones. GvHD is usually treated with corticosteroids and other immunosuppressants which do not always succeed in arresting its evolution. Extracorporeal photochemotherapy has been used in patients with both acute and chronic GvHD.